civil war: when the body attacks itself

Wednesday, August 31, 2011

The body seems to be under almost constant attack from the outside world, whether it is from harsh climate, from the sun’s ultraviolet rays or the bacteria and viruses that are everywhere. It seems especially unfair when the body attacks itself and causes illness. Autoimmune diseases can affect almost anyone and the latest patient in the spotlight is Venus Williams who withdrew from the US Tennis Open because of Sjogrens Syndrome.

“I’m really disappointed to have to withdraw from this year’s U.S. Open,” Williams said in a statement. I have recently been diagnosed with Sjogren’s Syndrome, an autoimmune disease which is an ongoing medical condition that affects my energy level and causes fatigue and joint pain…I enjoyed playing my first match here and wish I could continue but right now I am unable to. I am thankful I finally have a diagnosis and am now focused on getting better and returning to the court soon.” – Venus Williams in USA Today

Imagine what happens when lymphocytes, a type of white blood cell, decide that organs in the body are alien and don’t belong. They mount an attack and invade and infiltrate that organ causing it to fail. That is autoimmune disease in a nutshell. In Sjogrens Syndrome, the lymphocytes attack exocrine glands whose secretions through a duct directly to the outside of the body. The most common complaint is dry eyes and dry mouth since the glands that make tears and saliva are affected. {It’s important to remember that most people with dry eyes and mouth have it because of other, non-serious causes). Sjogrens would be no big deal if those were the only issues but the syndrome can also involve the lungs, kidney, liver and skin. As well, inflammation n of small blood vessels can result in Raynaud’s phenomenon and irritation of nerves can cause peripheral neuropathies.

There are plenty of theories as to what causes the autoimmune attack to start and there may be relationships to rheumatoid arthritis, systemic lupus erythematosis and scleroderma. The diagnosis is often challenging because patients often present with non-specific complaints like fatigue, joint ache and swelling abdominal pain and diarrhea as well as the classic dry eyes and dry mouth. It’s also a tough diagnosis because there isn’t total agreement as to how to make the diagnosis. There can be abnormal blood tests including autoantibodies (RF= rheumatoid factor and ANA=antinuclear antibody), and non- specific tests looking for inflammation like an ESR or erythrocyte sedimentation rate. There is an antibody that can be measured against the Sjogren Syndrome antigen but it is only positive in half the cases making the test less than helpful. Biopsies of salivary glands can be done to help confirm that lymphocytes have invaded the gland. There are consensus guidelines for making the diagnosis but mostly, it comes down to the doctor having a high index of suspicion and pursuing it.

If only the eyes and mouth are involved then treatment is aimed to control symptoms but aggressive medications may be needed if major organs like the lung, liver and kidney are involved. Another worry though is that more than 4% of patients develop non Hodgkin lymphoma that can present within 7-8 years of the initial diagnosis of Sjogrens. Most patients however do well and their prognosis is based on whether other autoimmune diseases like SLE are also involved.

When an athlete develops an uncommon disease, the only upside is the publicity and awareness that it brings. Hopefully, Ms. Williams will continue to perform as an elite athlete and perhaps some of her fans may see in her symptoms their own situation. Since the criteria to make the diagnosis are vague, it is uncertain just how many people suffer from Sjogrens Syndrome. It may take a star to put the disease into the spotlight.

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